Hypogenetic right lung with partial anomalous pulmonary venous return and accessory diaphragm: a case of “scimitar lung”

Melovitz-Vasan, Cheryl; White, Amanda; Huff, Susan; Vasan, Nagaswami

doi:10.5603/fm.a2022.0098

Cited by 1 publication

(1 citation statement)

References 25 publications

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“…The clinical presentation, constellation of symptoms, and overall severity of PAPVR are dependent on the amount of involved pulmonary veins and the degree of anomalous venous return. The spectrum of disease burden with PAPVR is vast and can range from completely asymptomatic to severe cardiopulmonary complications such as hypogenetic lung syndrome (Scimitar syndrome) resulting in global hypoplasia of a lung (typically the right lung) due to complete aberrant venous drainage from all involved pulmonary lobes [6]. As the burden of symptoms varies greatly with each patient's anatomy, with PAPVR, the various treatment options range from symptomatic management to complicated and complex open cardiothoracic and vascular surgery with the most notable being the Warden procedure [7].…”

Section: Introductionmentioning

confidence: 99%

Intraoperative Acute Cardiac Tamponade as a Result of Intracardiac Perforation Requiring Emergency Continuous Pericardiocentesis and Open Sternotomy: A Case Report and Literature Review of a Rare but Fatal Complication

Russo,

Harrison,

Harry

et al. 2024

Cureus

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Intraoperative acute cardiac tamponade associated with iatrogenic intracardiac perforation from percutaneous interventional cardiac procedures is a rare but potentially catastrophic complication. We report a case of intraoperative acute hemopericardium caused by a left atrial (LA) perforation resulting in cardiac tamponade in a patient undergoing a baffling procedure for the correction of two anomalous pulmonary veins draining into her superior vena cava (SVC) that required continuous pericardiocentesis with autologous blood transfusion via the femoral vein and an emergency intraoperative transfer from the interventional cardiology cath lab to the cardiac operating room for an open sternotomy and primary repair. An 86-year-old female with known right-ventricular (RV) failure with preserved ejection fraction (left ventricular ejection fraction (LVEF): 50-55% on transesophageal echocardiography (TEE) one week prior) and atrial fibrillation was admitted for her third heat failure exacerbation in two months despite being adherent to her aggressive diuresis medication regimen. Upon her readmission and due to her symptomatic and seemingly refractory heart failure, the patient underwent a cardiac computer tomography (CT) with 3D reconstruction that showed previously undiagnosed partial anomalous pulmonary venous return (PAPVR) of two of her four pulmonary veins aberrantly draining into the SVC. This anatomic pathology was deemed to be the likely etiology of her repeated episodes of recurring heart failure exacerbations, shortness of breath, peripheral edema, and fatigue. The patient was counseled and consented to a percutaneous baffle of the two anomalous veins to redirect more of the returning pulmonary venous blood away from the SVC and to the LA. While under general endotracheal anesthesia (GETA) with a TEE in place during the procedure, the patient suddenly developed acute hypotension, tachycardia, and a reduction in expired carbon dioxide (EtCO 2 ) was noted quickly followed by evidence of a rapidly accumulating hemopericardium on TEE.Cardiothoracic surgery was urgently consulted to the interventional cardiology cath lab while the patient underwent an emergency pericardiocentesis that momentarily alleviated her hemodynamic instability, cardiac tamponade physiology, and deteriorating overall clinical picture. While performing continuous pericardiocentesis with autologous return of the aspirated blood via femoral venous access the patient was urgently transported to the cardiac operating room and prepped for emergency sternotomy for primary repair of the LA. Following primary repair via sternotomy, multiple drains were placed and the thoracic cavity was closed with wires. The patient was immediately transported to the surgical intensive care unit (SICU) intubated, mechanically ventilated, and sedated. During this time, the patient progressively required additional vasoactive and inotropic agents to support her mean arterial pressure (MAP), and following a multidisciplinary discussion with the patient's family regardi...

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Section: Introductionmentioning

confidence: 99%

Intraoperative Acute Cardiac Tamponade as a Result of Intracardiac Perforation Requiring Emergency Continuous Pericardiocentesis and Open Sternotomy: A Case Report and Literature Review of a Rare but Fatal Complication

Russo,

Harrison,

Harry

et al. 2024

Cureus

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Hypogenetic right lung with partial anomalous pulmonary venous return and accessory diaphragm: a case of “scimitar lung”

Abstract: This article has been peer reviewed and published immediately upon acceptance.It is an open access article, which means that it can be downloaded, printed, and distributed freely, provided the work is properly cited. Articles in "Folia Morphologica" are listed in PubMed.

Cited by 1 publication

References 25 publications

Intraoperative Acute Cardiac Tamponade as a Result of Intracardiac Perforation Requiring Emergency Continuous Pericardiocentesis and Open Sternotomy: A Case Report and Literature Review of a Rare but Fatal Complication

Intraoperative Acute Cardiac Tamponade as a Result of Intracardiac Perforation Requiring Emergency Continuous Pericardiocentesis and Open Sternotomy: A Case Report and Literature Review of a Rare but Fatal Complication

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